Klippel Trenaunay Syndrome

What is Klippel Trenaunay Syndrome?

A rare and genetic syndrome that is often the most severe manifestations of varicose vein disease. The syndrome is a vascular disorder that consists of abnormalities in the venous system, lymphatic system, and arterial systems. This disorder often manifests itself in the form of varicose veins, arterio-venous malformations (AVMs), lymphedema, port-wine stains and eventually limb hypertrophy (enlargement of the involved extremity).

Treatment

Treatment options are quite limited in this syndrome given the extent of the disease present in the affected limb. Since Klippel Trenaunay is a syndrome, it varies from person to person. Therefore, the treatment options available will depend on what abnormalities are present in the affected limb. For those patients who have lymphedema, treatment will focus on chronic compression with either graduated compression stockings or circaids compression garments. Lymphedema physical therapy may also be used in conjunction with the compression devices to form the foundation of the treatment. For those patients who possess venous abnormalities, standard vein treatment modalities like endovenous laser ablation (ELT), ultrasound guided and visually guided sclerotherapy can also be used to help control the venous disease component of a patient’s Klippel Trenaunay syndrome. In cases of AVM’s, treatment options include either injecting the AVM’s with sclerosant to force the vessels to close down if the AVM is small or if the AVM is larger, conservative treatment may be the treatment of choice. Klippel Trenaunay syndrome is a very complex disorder due to its impact on multiple vascular systems. Caution and conservative treatment are the operative words when treating patients afflicted with Klippel Trenaunay syndrome.